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Hypokalemic periodic paralysis (HypoPP) is the most prevalent form of familial periodic paralysis and classically presents with recurrent attacks of moderate to severe generalized weakness in association with hypokalemia (often <3.0 meq/L). As with all forms of periodic paralysis, the defect in force generation in HypoPP stems from a reduction or complete loss of muscle excitability. Affected fibers are persistently depolarized, which inactivates muscle sodium channels and thereby prevents the fibers from generating a propagated action potential. The molecular basis for this electrical catastrophe originates in mutations of genes encoding ion channels of skeletal muscle.1 Remarkably, mutations of either the voltage-gated sodium channel or the voltage-gated calcium channel can cause clinically indistinguishable HypoPP. In this issue of Neurology®, Matthews et al.2 provide compelling evidence that missense mutations of arginine residues in specialized voltage-sensing regions of the two different ion channels are a common theme in HypoPP.
Over the past 2 decades, it has been firmly established that the many variants of familial periodic paralysis and …
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